Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001383042 | SCV001582053 | pathogenic | not provided | 2024-07-17 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with leucine, which is neutral and non-polar, at codon 243 of the CAPN5 protein (p.Arg243Leu). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individual(s) with autosomal dominant neovascular inflammatory vitreoretinopathy (PMID: 23055945). It has also been observed to segregate with disease in related individuals. ClinVar contains an entry for this variant (Variation ID: 39806). Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is not expected to disrupt CAPN5 protein function with a negative predictive value of 80%. Experimental studies have shown that this missense change affects CAPN5 function (PMID: 23055945, 24381307, 25994508). For these reasons, this variant has been classified as Pathogenic. |
OMIM | RCV000033027 | SCV000056807 | pathogenic | Proliferative vitreoretinopathy | 2012-01-01 | no assertion criteria provided | literature only | |
Genome |
RCV001535499 | SCV001749452 | not provided | Autosomal dominant neovascular inflammatory vitreoretinopathy | no assertion provided | phenotyping only | Variant interpreted as Pathogenic and reported on 11-05-2020 by Invitae. GenomeConnect-Invitae Patient Insights Network assertions are reported exactly as they appear on the patient-provided report from the testing laboratory. Registry team members make no attempt to reinterpret the clinical significance of the variant. Phenotypic details are available under supporting information. |